Imaging Pearls ❯ Liver ❯ Cholangiocarcinoma
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- “Cholangiocarcinoma (CCA) is the second most common primary hepatobiliary malignancy and presents as three separate morphological subtypes; namely mass-forming, periductal-infiltrating, and intraductal-growing patterns. Each of these subtypes have distinct imaging characteristics, as well as a variety of benign and malignant mimics, making accurate diag- nosis of CCA on imaging challenging. Whilst histopathological examination is required to arrive at a definitive diagnosis, it is still important for radiologists to be cognizant of these entities and provide reasonable differential diagnoses, as these potentially have a large impact on patient management.”
Imaging mimickers of cholangiocarcinoma: a pictorial review
Si Min Chiow et al.
Abdominal Radiology (2022) 47:981–997 - "Morphologically, CCA can show three main growth patterns: mass-forming, periductal-infiltrating, and intra- ductal-growing patterns. Mass-forming CCA manifests as a nodular mass invading into the hepatic parenchyma. Periductal-infiltrating CCA appears as an infiltrative lesion growing along the bile duct wall and spreads via the perineural tissue and lymphatic vessels of the Glisson sheath. Finally, intraductal-growing CCAs are macroscopically visible exophytic tumours within the bile duct lumen. Intraductal growing CCA are associated with longer survival compared to the other forms of CCA.”
Imaging mimickers of cholangiocarcinoma: a pictorial review
Si Min Chiow et al.
Abdominal Radiology (2022) 47:981–997 - “Mass-forming CCA usually appears as a homogeneous low-attenuating mass on non-contrast-enhanced CT, and a T1W iso-/hypo-intense and T2W hyperintense mass on MRI. Additional findings may include capsular retraction, dila- tion of peripheral bile ducts, and satellite nodules. Although less common compared to HCC, mass- forming CCA can also show vascular invasion. Indeed, a recent meta-analysis by Kim et al. showed that CCA is the third most common cause of tumour-in-vein after HCC and cHCC-CCA.
Imaging mimickers of cholangiocarcinoma: a pictorial review
Si Min Chiow et al.
Abdominal Radiology (2022) 47:981–997 - Imaging mimickers of cholangiocarcinoma (mass-like)
- Hepatocellular carcinoma (HCC)
- Combined hepatocellular cholangiocarcinoma
- Sclerosed haemangioma
- Confluent hepatic fibrosis
- Lymphoma
- Adenocarcinoma metastases
Imaging mimickers of cholangiocarcinoma: a pictorial review
Si Min Chiow et al.
Abdominal Radiology (2022) 47:981–997 - Imaging mimickers of cholangiocarcinoma (infiltrating)
- IgG4‐related disease
- Benign fibrosis
- Primary sclerosing cholangitis (PSC)
- Gallbladder carcinoma with extension to hilum
- Tuberculosis (TB)
- Intraductal papillary neoplasm of the bile duct (IPNB)
Imaging mimickers of cholangiocarcinoma: a pictorial review
Si Min Chiow et al.
Abdominal Radiology (2022) 47:981–997
- “Moreover, radiomics-predicted lymph node metastasis emerged as a preoperative predictor of both disease-specific survival and recurrence-free survival after curative intent resection of biliary tract cancers (hazard ratios, 3.37 and 1.98, respectively). Overall, there was important personalized information for medical decision support.”
CT-based Radiomics for Biliary Tract Cancer: A Possible Solution for Predicting Lymph Node Metastases
Laghi A, Voena C
Radiology 2019; 290:99–100 - There are limitations. Although the model was built with rigorous methodologic structure, a multicentric study collecting a larger number of patients would be necessary to check for the generalizability of the radiomics signature. The influence of different CT parameters (eg, kilovolt, milliampere-seconds, and reconstruction filters) on extraction of radiomics features was not among the objectives of this study, although this is a relevant variable that might affect data consistency and limit the extensive use of the model.
CT-based Radiomics for Biliary Tract Cancer: A Possible Solution for Predicting Lymph Node Metastases
Laghi A, Voena C
Radiology 2019; 290:99–100 - A correlation with genomic profile of biliary tract cancers may have been desirable, especially in the era of target therapy where specific genomic profiles are associated with either response or resistance to a specific drug. Nevertheless, radiomics approaches seem to have a bright future, especially if collaborative multidisciplinary teams are involved. Ultimately, to achieve personalized medicine, personalized imaging must be involved.
CT-based Radiomics for Biliary Tract Cancer: A Possible Solution for Predicting Lymph Node Metastases
Laghi A, Voena C
Radiology 2019; 290:99–100 - “Ultimately, to achieve personalized medicine, personalized imaging must be involved."
CT-based Radiomics for Biliary Tract Cancer: A Possible Solution for Predicting Lymph Node Metastases
Laghi A, Voena C
Radiology 2019; 290:99–100 - “The further goal of radiomics analytics is to develop decision support tools, such as predictive models, by incorporating radiomics signature and other morphologic features. Radiomics models providing individualized risk estimation of LN metastasis have been developed and validated in studies focused on esophageal, colorectal, and bladder cancers with good results."
CT-based Radiomics for Biliary Tract Cancer: A Possible Solution for Predicting Lymph Node Metastases
Laghi A, Voena C
Radiology 2019; 290:99–100
- “Cholangiocarcinoma is the second most common primary malignancy of the liver and the most common biliary tract tumor, with the highest frequency in Southeast Asia. Cholangiocarcinoma constitutes 10–15% of primary hepatic cancers and can be categorized as extrahepatic, peripheral intrahepatic, and hilar intrahepatic. Intrahepatic cholangiocarcinomas stem from the biliary system peripheral to the secondary bifurcation of the left or right hepatic ducts and are divided into three categories on the basis of their growth pattern.”
Liver Calcifications and Calcified Liver Masses: Pattern Recognition Approach on CT Madhavi Patnana et al AJR 2018; 211:76–86
- “ICC (Intrahepatic cholangiocarcinoma)
can be classified on the basis of the macroscopic tumor growth pattern as mass- forming type, periductal infiltrating type, or intraductal growing type according to the classification of the Liver Cancer Study Group of Japan. The mass-forming type is the most common, accounting for 78% of all cases of ICC.”
Cross-Sectional Imaging of Intrahepatic Cholangiocarcinoma: Development, Growth, Spread, and Prognosis Seo N et al. AJR 2017; 209:W64–W75 - “The mass-forming type is the most common, accounting for 78% of all cases of ICC. Tumors of this type are usually large, up to 15 cm in diameter. The majority manifest as well-defined lobulated masses with varying degrees of central sclerotic changes. Multicentricity around the main tumor is common, probably because mass-forming ICC commonly invades the adjacent portal vein branches.”
Cross-Sectional Imaging of Intrahepatic Cholangiocarcinoma: Development, Growth, Spread, and Prognosis Seo N et al. AJR 2017; 209:W64–W75 - “Periductal infiltrating tumors extend lon-
gitudinally along the bile ducts and cause bile duct wall thickening. Progressive periductal invasion causes luminal stenosis and proximal biliary dilatation. Although periductal infiltrating type is the most common type of hilar cholangiocarcinoma, it is much less common in ICC, constituting approximately 16% of ICCs.”
Cross-Sectional Imaging of Intrahepatic Cholangiocarcinoma: Development, Growth, Spread, and Prognosis Seo N et al. AJR 2017; 209:W64–W75 - “Intraductal growing ICC is the rarest type of ICC (approximately 6%) and presents as a papillary tumor within the dilated bile duct lumen; this type shares morphologic features with intra- ductal papillary neoplasm of the bile duct (IPNB). Intraductal growing ICCs are usually small, sessile, or polypoid and spread along the mucosa with multiplicity.”
Cross-Sectional Imaging of Intrahepatic Cholangiocarcinoma: Development, Growth, Spread, and Prognosis Seo N et al. AJR 2017; 209:W64–W75
- "Cholangiocarcinoma is a heterogeneous tumor classified by location as intrahepatic (ICC), perihilar (PCC), and distal (DCC). The terms extrahepatic and Klatskin tumor, referring to perihilar tumors, are now discouraged. ICC arises distal to the second-order bile ducts. PCC is proximal to the second biliary bifurcation, and DCC is distal to the cystic duct insertion. ICC is the second most common primary malignant hepatic tumor."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "The incidence of cholangiocarcinoma varies depending on geography due to different risk factors. Southeast Asian countries such as Thailand have the highest reported incidence of cholangiocarcinoma (113 per 100,000) due to the prevalence of parasitic infections such as Opisthorchis viverrini resulting in chronic inflammation. Another hepatobiliary fluke such as Clonorchis sinensis has a similar association in South Korea. Hepatolithiasis is also an established risk factor in Asian countries."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "Primary sclerosing cholangitis (PSC) and choledochal cysts are also major risk factors for cholangiocarcinoma. Thorotrast exposure is another strong risk factor. Other associations include inflammatory bowel disease, choledocholithiasis, chronic viral hepatitis, cirrhosis, diabetes, alcohol exposure, smoking, obesity, and genetic polymorphisms."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "Choledochal cysts are congenital disorders that often develop from an abnormal pancreatico-biliary junction with a 20-30 fold increase in risk of developing cholangiocarcinoma at a mean age of 32 years. In a choledochal cyst, cholangiocarcinoma will appear as wall irregularity with or without a mass, or papillary nodules. Although screening magnetic resonance imaging (MRI) is thought to be helpful in the early detection of cholangiocarcinoma, there are no established guidelines."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "On CT, mass-forming ICC usually appears as a pre- dominantly hypodense mass with a lobulated contour. Irregular peripheral enhancement is visualized during arterial and portal venous phases with progressive central enhancement during delayed phase. Due to this appearance, cholangiocarcinoma can be mistaken for a hemangioma. Delayed enhancement is directly proportional to the amount of interstitial space in the fibrous stroma. Necrosis and mucin can contribute to an absence of delayed enhancement. The early enhancing periphery of the mass represents rapidly growing tumor cells. The dense fibrous nature of the tumor leads to capsular retraction in approximately 1/3 of tumors ."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "Intraductal tumor in contrast is typically hypoenhancing. Bile ducts are expanded by hypodense or hypointense mucin . IPNB compared to intraductal papillary cholangiocarcinoma has multiple papillary masses rather than a single nodule, biliary dilatation proximal and distal to the masses, and hypoenhancement rather than delayed hyperenhancement. IPNB also can be seen to be growing along the interior of the bile duct rather than invading the bile duct wall ."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "On CT, an abrupt change in caliber of the duct indicates the obstructing mass. A mass may or may not be seen, depending on tumor morphology. Malignant biliary thickening is typically longer than 5 mm and more irregular. Ductal dilatation usually has a segmental or lobar distribution. Hepatic lobar atrophy may result from biliary or portal vein occlusion. Mini- mum intensity projection images may also be used to better depict the biliary system."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - "Up to 26% of hepatic resections for presumed cholangiocarcinoma are negative for tumor. Most mimics are inflammatory or infectious, including IgG4 cholangiopathy, PSC, recurrent pyogenic cholangitis, Mirizzi syndrome, and HIV cholangiopathy. Malignant strictures are usually longer, more irregular, and asymmetric. The majority of malignant strictures are arterially hyperenhancing and the majority of benign strictures are arterially isoenhancing or hypoenhancing."
Imaging spectrum of cholangiocarcinoma: role in diagnosis, staging, and posttreatment evaluation
Mar WA et al.
Abdom Radiol (2016) 41:553-567 - Capsular Retraction of the Liver: Differential Dx
- Cholangiocarcinoma
- Metastases
- Epithelioid hemangioendothelioma
- Fibrolamellar HCC
- Cholangiocarcinoma
- Tumor arises from the bile duct epithelium
- 95% adenocarcinoma
- Associated with:
- PSC
- Choledochal cysts
- Drug exposures (such as thoratrast)
- Congenital hepatic fibrosis
- Viral hepatitis - Cholangiocarcinoma
- Intrahepatic
- Mass forming intrahepatic cholangiocarcinoma
- Periductal infiltrating cholangiocarcinoma
- Intraductal cholangiocarcinoma
- Hilar Cholangiocarcinoma
- Extrahepatic Cholangiocarcinoma - Cholangiocarcinoma
- Variable appearance
- Peripheral hypervascularity on arterial phase images (and sometimes venous phase)
- Hypovascular lesions
- Delayed enhancement
- Dilated bile ducts (with bile duct thickening)
- Capsular retraction
- Lobar or segmental atrophy
- Cholangiocarcinoma
- Tumor arises from the bile duct epithelium
- 95% adenocarcinoma
- Associated with:
- PSC
- Choledochal cysts
- Drug exposures (such as thoratrast)
- Congenital hepatic fibrosis
- Viral hepatitis - Cholangiocarcinoma
1. Intrahepatic
- Mass forming intrahepatic cholangiocarcinoma
- Periductal infiltrating cholangiocarcinoma
- Intraductal cholangiocarcinoma
2. Hilar Cholangiocarcinoma
3. Extrahepatic Cholangiocarcinoma - Cholangiocarcinoma
- Variable appearance
- Peripheral hypervascularity on arterial phase images (and sometimes venous phase)
- Hypovascular lesions
- Delayed enhancement
- Dilated bile ducts (with bile duct thickening)
- Capsular retraction
- Lobar or segmental atrophy